Department of Neurology, National Hospital of Niamey, Niamey, Niger.
Department of Neurology, Hassan II University Teaching Hospital, Fez, Morocco.
Brain Behav. 2019 Jan;9(1):e01177. doi: 10.1002/brb3.1177. Epub 2018 Nov 25.
Histologically defined as an inflammation-degeneration of limbic structures, limbic encephalitis (LE) is a rare disease and often difficult to diagnose particularly in institutions with limited access to laboratory tests such as antineuronal antibodies or HSV-PCR, and functional imaging. We aimed to describe the demographic, clinical, paraclinical, and etiological features of LE, as well as its medium-term prognosis in Moroccan patients.
We collected retrospectively all patients diagnosed with LE in the Department of Neurology of the University Hospital Hassan II of Fez (Morocco) between September 2008 and December 2016. We analyzed their demographic features, clinical manifestations, magnetic resonance imaging and laboratory findings, etiologies, and medium-term prognoses.
We included 22 men and 9 women aged 14-76 years (mean age: 45.8 years). In 64.5% of cases, the onset of symptoms was acute. The clinical manifestations included generalized status epilepticus (16.1%), confusional syndrome (29%), epileptic seizures (38.7%), psychiatric disorders (48.4%), and memory disorders (45.2%). The nonlimbic symptoms were nuchal stiffness (22.6%), headaches (9.7%), fever (61.3%), vesicular rash (3.2%), and language disorders (6.5%). The different etiologies found were herpes simplex virus (6.5%), syphilis (16.1%), tuberculosis (3.2%), varicella (3.2%), paraneoplastic autoimmune LE (22.6%), anti-NMDA-R LE (6.5%), and sarcoidosis (3.2%). We found 12 cases (38.7%) of LE without definite etiology and with an incomplete diagnostic workup. The medium-term clinical course includes a complete remission in 45.2% of cases and partial remission in 45.1% of cases. The different sequelae were temporal lobe epilepsy (9.7%), anterograde amnesia (16.1%), and severe cognitive impairment (19.4%). The mortality rate was 9.7% (3 patients).
Our study shows a wide diversity of etiologies of LE in Morocco with essentially an acute mode of onset of symptoms.
从组织学上定义为边缘结构的炎症-变性，边缘性脑炎（LE）是一种罕见疾病，尤其是在实验室检测（如神经元抗体或 HSV-PCR 检测和功能成像）受限的机构中，常常难以诊断。我们旨在描述摩洛哥患者 LE 的人口统计学、临床、辅助检查和病因特征及其中期预后。
我们回顾性地收集了 2008 年 9 月至 2016 年 12 月期间在摩洛哥哈桑二世大学医院神经科诊断为 LE 的所有患者。我们分析了他们的人口统计学特征、临床表现、磁共振成像和实验室检查、病因以及中期预后。
我们纳入了 22 名男性和 9 名女性，年龄 14-76 岁（平均年龄：45.8 岁）。64.5%的病例起病急骤。临床表现包括全身性癫痫持续状态（16.1%）、意识模糊综合征（29%）、癫痫发作（38.7%）、精神障碍（48.4%）和记忆障碍（45.2%）。非边缘性症状包括颈项强直（22.6%）、头痛（9.7%）、发热（61.3%）、疱疹样皮疹（3.2%）和语言障碍（6.5%）。发现的不同病因包括单纯疱疹病毒（6.5%）、梅毒（16.1%）、结核（3.2%）、水痘（3.2%）、副肿瘤自身免疫性 LE（22.6%）、抗-NMDA-R LE（6.5%）和结节病（3.2%）。我们发现 12 例（38.7%）无明确病因且诊断性检查不完整的 LE 病例。中期临床病程包括完全缓解（45.2%）和部分缓解（45.1%）。不同的后遗症包括颞叶癫痫（9.7%）、顺行性遗忘（16.1%）和严重认知障碍（19.4%）。死亡率为 9.7%（3 例）。
我们的研究表明，摩洛哥的 LE 病因广泛多样，主要表现为症状急性起病。
